Extracranial hypoglossal neurofibroma with a variant ansa cervicalis: a case report

Surg Radiol Anat. 2023 Mar;45(3):297-302. doi: 10.1007/s00276-023-03085-z. Epub 2023 Feb 1.

Abstract

Purpose: This case report aims to explore a rare combination of findings in a cadaver donor: variant ansa cervicalis, vagus (CN X) and hypoglossal (CN XII) nerve fusion, and extracranial hypoglossal neurofibroma.

Background: The type of ansa cervicalis variation presented in this report has been documented in less than 1% of described cases. The CN X-CN XII fusion has been reported in one prior study. Additionally, hypoglossal neurofibromas are benign neoplasms of the peripheral nerve sheath. There are only two known cases of extracranial hypoglossal neurofibroma described in the literature.

Case report: The study investigated a swelling of the right CN XII in a 90-year-old female cadaver donor. Detailed dissection, examination of the region, and histopathological analysis of the mass followed. The entire course of CN XII and other cranial nerves were examined to exclude concurrent pathology. A fusiform enlargement of the right CN XII was observed in the submandibular region, measuring ~ 1.27 × 1.27 cm. The superior portion of the right CN XII was fused to the right CN X, exiting the jugular foramen. The superior root of ansa cervicalis, normally a branch of CN XII, was found to arise from CN X on the right side. The left CN XII and CN X were unremarkable. Histopathological examination revealed benign neurofibroma.

Conclusion: The anatomical variation and rare location of the tumor necessitate further investigation to better understand pathogenesis, clinical correlation, and surgical implications. This study furthers knowledge of this condition and contributes to the currently limited body of research.

Keywords: Anatomical variation; Ansa cervicalis; Extracranial neurofibroma; Neurofibroma.

Publication types

  • Case Reports

MeSH terms

  • Aged, 80 and over
  • Cadaver
  • Cervical Plexus* / anatomy & histology
  • Dissection
  • Female
  • Humans
  • Hypoglossal Nerve / anatomy & histology
  • Neurofibroma* / diagnosis
  • Neurofibroma* / surgery
  • Vagus Nerve